Necrobiotic xanthogranuloma with amyloidosis of the skin and the liver

Background: Necrobiotic xanthogranuloma is a largely unknown disease. Both the frequency of this disease and the involvement of internal organs have c learly been underestimated until now. Case Report: A patient was admitted because of ulcerating, xanthomatous, su bcutaneous nodules, scleritis and conjunctivitis. Laboratory studies reveal ed an elevated erythrocytes sedimentation rate, a monoclonal IgG-lambda pro tein, a pancytopenia and later a hypocompletementemia. Furthermore, a hepat osplenomegaly and esophageal varices were found. A skin biopsy specimen sho wed a granulomatous infiltrate consisting of lymphocytes, histiocytes, plas ma cells, foam cells as well as Touton and bizarre-appearing foreign-body g iant cells and cholesterol clefts typical of necrobiotic xanthogranuloma be sides a deposit of amyloid. A liver biopsy sample disclosed an amyloidosis of the parenchyma, too. Neither a therapy with chlorambucil and prednisolon e nor with interferon alpha-2a resulted in improvement. Conclusion: Apart from treatment of skin lesions and ophthalmic manifestati ons further investigations are necessary because necrobiotic xanthogranulom a can be associated with malignancy and can involve internal viscera like l ungs, heart and liver.